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Vitreitis Secondary to CNS Lymphoma

HomeIntraocular Inflammation and Uveitis / Masquerade Syndromes /


Title:
Vitreitis Secondary to CNS Lymphoma

Authors: Michael Simmons, BS

Photographer: Melissa Chandler

Date: 07/27/2016

On presentation, the patient had disc edema and a small optic nerve hemorrhage at 2 o’clock.

On presentation, the patient had disc edema and a small optic nerve hemorrhage at 2 o’clock.

Fluorescein angiography demonstrated leakage from the optic nerve. Vitreitis was also noted.

Fluorescein angiography demonstrated leakage from the optic nerve. Vitreitis was also noted.

Keywords / Main Subjects: CNS lymphoma; Disc edema; vitreitis

Diagnosis: Central nervous system extranodal natural killer cell/t-cell lymphoma (ENKTL) with optic nerve involvement and left vitreitis

Brief Description of Image: Extranodal natural killer/T-cell lymphoma (ENKTL) is a type of non-Hodgkin lymphoma [1]. It makes up approximately 1% of all lymphomas in the US, but may occur more frequently in Hispanics and Native Americans[2]. It has a close association with Epstein-Barr virus infection and typically manifests in the nasal cavity [3]. ENKTL is an aggressive disease with a poor prognosis [4]. There is a tendency to metastasize to the central nervous system [2]. Although rare, ocular involvement has been noted[5][6]. Ocular symptoms are nonspecific, and include such entities as eyelid edema and ptosis, blurred vision, optic nerve swelling, and steroid refractory anterior uveitis and vitreitis [2,5,7].

The patient is a 38-year-old man with a 26-year history of extranodal natural killer cell/T-cell lymphoma with multiple recurrences including central nervous system recurrences despite allogeneic bone marrow transplant. He presented for evaluation of six weeks of floaters and a new blind spot in his left eye. On exam, he was found to have diminished visual acuity with a 0.6 log left afferent pupillary defect, but color vision testing with Ishihara plates and a flicker fusion test were normal. He was found to have an intraocular pressure in his left eye. On slit lamp exam, there were 2+ cells in the left anterior chamber and 3+ vitreous cells with a pre-papillary and pre-macular vitreous infiltrate. There was also grade II disc edema.  Fluorescein angiography showed no evidence of retinitis, but there was bilateral disc leakage, more prominent on the left than the right.

Flow cytometry performed on the patient’s cerebrospinal fluid revealed 20% abnormal natural killer cells lacking CD16 with prominent expression of CD57. Labs investigating alternate etiologies, including tuberculosis, syphilis, toxoplasmosis, bartonellosis, Lyme disease and sarcoidosis were negative. Previous therapies included chemotherapy, radiation therapy, infusions of EBV-specific T-cells, and allogeneic bone marrow transplant. With his current relapse, the patient received palliative external radiation therapy of the head and intrathecal cytarabine. The team also renewed discussions regarding hospice and end-of-life care.

References:

  1. Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, et al. World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting-Airlie House, Virginia, November 1997. J Clin Oncol. 1999;17: 3835–3849.
  2. Cimino L, Chan C-C, Shen D, Masini L, Ilariucci F, Masetti M, et al. Ocular involvement in nasal natural killer T-cell lymphoma. Int Ophthalmol. 2009;29: 275–279.
  3. Li S, Feng X, Li T, Zhang S, Zuo Z, Lin P, et al. Extranodal NK/T-cell lymphoma, nasal type: a report of 73 cases at MD Anderson Cancer Center. Am J Surg Pathol. 2013;37: 14–23.
  4. Hatta C, Ogasawara H, Okita J, Kubota A, Ishida M. Non-Hodgkin’s malignant lymphoma of the sinonasal tract—treatment outcome for 53 patients according to REAL classification. Auris Nasus Larynx. Elsevier; 2001; Available: http://www.sciencedirect.com/science/article/pii/S0385814600000948
  5. Abe RY, Pinto RDP, Bonfitto JFL, Lira RPC, Arieta CEL. Ocular masquerade syndrome associated with extranodal nasal natural killer/T-cell lymphoma: case report. Arq Bras Oftalmol. 2012;75: 430–432.
  6. Maruyama K, Kunikata H, Sugita S, Mochizuki M, Ichinohasama R, Nakazawa T. First case of primary intraocular natural killer t-cell lymphoma. BMC Ophthalmol. 2015;15: 169.
  7. Yoo JH, Kim SY, Jung KB, Lee JJ, Lee SJ. Intraocular involvement of a nasal natural killer T-cell lymphoma: a case report. Korean J Ophthalmol. 2012;26: 54–57.

Disclosure (Financial or other): None

Identifier: Moran_CORE_21679

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